A nine-month-old boy was admitted to our hospital with hepatomegaly and precocious puberty. Ultrasonography, CT-scan and DSA-angiography demonstrated a huge tumor in the right lobe of the liver. Serum AFP was 877400ng/ml and Human Chorionic Gonadotropin (HCG) level in serum and urine were 36IU/ml and 66MIU/ml. The patient underwent a right hepatic lobectomy. Histology of the resected tumor revealed embryonal type hepatoblastoma and HCG in the multinucleated giant cells in the immunohistochemically (PAP method). HCG in serum normalized at day 8 after surgery, and serum AFP fell progressively to a 14ng/ml. The patient underwent treatment of chemotherapy during the following 3 months. The boy is asymptomatic with two years follow-up and no tumoral recurrence.